Idh2 Gene plays an important role in the study of neurodegenerative diseases. This page provides comprehensive information about this topic, including its mechanisms, significance in disease processes, and therapeutic implications.
Idh2 Gene is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
{{Infobox gene
| symbol = IDH2
| name = Isocitrate Dehydrogenase 2
| chromosome = 15
| locus = 15q26.1
| geneID = 3416
| omim = 147650
| ensembl = ENSG00000182054
| uniprot = P00342
| uniprot_name = IDH2
| diseases = Amyotrophic Lateral Sclerosis, Alzheimer's Disease, 2-Hydroxyglutaric Aciduria
| diseases_ref = Tannous et al., 2018, Nat Neurosci
}}
Isocitrate dehydrogenase 2 (IDH2) is a mitochondrial enzyme that catalyzes the oxidative decarboxylation of isocitrate to α-ketoglutarate (α-KG), producing NADPH in the mitochondrial matrix. IDH2 is one of three IDH isoforms in humans and is primarily localized to mitochondria.
IDH2 is a key enzyme in the TCA cycle, catalyzing: Isocitrate + NAD(P)+ → α-Ketoglutarate + NAD(P)H + H+ + CO2. The enzyme requires Mg2+ or Mn2+ as a cofactor and is allosterically activated by ADP and inhibited by ATP and NADPH.
IDH2 is a major source of mitochondrial NADPH, which is essential for:
Through α-ketoglutarate production, IDH2 influences:
IDH2 mutations are found in some ALS patients. Reduced IDH2 activity leads to:
IDH2 activity decreases with age and in AD brains. Lower IDH2 leads to:
IDH2 deficiency causes accumulation of D-2-hydroxyglutarate, which:
IDH2 is expressed in:
In the brain, IDH2 is highly expressed in:
Idh2 Gene plays an important role in the study of neurodegenerative diseases. This page provides comprehensive information about this topic, including its mechanisms, significance in disease processes, and therapeutic implications.
The study of Idh2 Gene has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
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Shay JW et al.. "Abstracts from the 3rd International Genomic Medicine Conference (3rd IGMC 2015) : Jeddah, Kingdom of Saudi Arabia. 30 November - 3 December 2015." BMC genomics (2016) DOI:10.1186/s12864-016-2858-0
Tang M et al.. "Hippocampal proteomic changes of susceptibility and resilience to depression or anxiety in a rat model of chronic mild stress." Translational psychiatry (2019) DOI:10.1038/s41398-019-0605-4
Sardoiwala MN et al.. "Hytrin loaded polydopamine-serotonin nanohybrid induces IDH2 mediated neuroprotective effect to alleviate Parkinson's disease." Biomaterials advances (2022) DOI:10.1016/j.msec.2021.112602
May JL et al.. "IDH3α regulates one-carbon metabolism in glioblastoma." Science advances (2019) DOI:10.1126/sciadv.aat0456
Mandal P et al.. "Mitochondrial bioenergetic signatures differentiate asymptomatic from symptomatic Alzheimer's disease." bioRxiv : the preprint server for biology (2025) DOI:10.1101/2025.11.04.686626
Pan JH et al.. "SIRT3-IDH2 axis is a target of dietary fructose: implication of IDH2 as a key player in dietary carcinogen toxicity in mice colon." Experimental & molecular medicine (2025) DOI:10.1038/s12276-025-01584-0
Chen WW et al.. "T817MA Regulates Mitochondrial Dynamics via Sirt1 and Arc Following Subarachnoid Hemorrhage." Neuroscience (2023) DOI:10.1016/j.neuroscience.2023.06.020